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[足月儿或近足月儿的局灶性自发性结肠穿孔:罕见且可能隐匿]

[Focal spontaneous colic perforation in term or near-term neonates: rare and potentially insidious].

作者信息

Bartoli F, Vasseur Maurer S, Giannoni E, Osterheld M-C, Laubscher B

机构信息

Département de pédiatrie, hôpital Neuchâtelois, rue de Maladière 45, 2000 Neuchâtel, Suisse.

出版信息

Arch Pediatr. 2011 Apr;18(4):408-12. doi: 10.1016/j.arcped.2011.01.004.

Abstract

Two cases of neonatal focal spontaneous colic perforations are reported. The 1st infant, born at 36 3/7 weeks gestational age, presented on day 3 with crying, abdominal distension, and liquid stools. Clinical examination showed a slightly irritable hypothermic (35.7 °C) infant with a distended abdomen and few bowel sounds. Blood tests were normal apart from an elevated C-reactive protein level (59 mg/l). The abdomen x-ray was erroneously considered normal. The infant's condition remained stable for nearly 3 days. After reviewing the initial x-ray, pneumoperitoneum was suspected and confirmed by a cross-table lateral abdominal x-ray. The infant was started on antibiotics and operated. Macroscopically, the entire gut was normal apart from a focal sigmoid perforation, which was stitched. A transmural colic biopsy revealed focal vascular dilation but was negative for necrotising enterocolitis or Hirschsprung disease. The infant recovered quickly. She is now a healthy, normal 3-year-old. The 2nd infant, born at 38 5/7 weeks gestational age, presented between day 1 and 2 with clinical signs of infection associated with slowly progressive ileus. The chest and abdomen x-ray was mistakenly considered normal. Frank septicemia developed. After reviewing the initial x-ray, pneumoperitoneum was suspected and confirmed by a cross-table lateral abdominal x-ray. The infant was operated. Macroscopically, the small intestine was normal, the ascending and transverse colons were dilated, and the descending and sigmoid colons were narrow. Three cecal perforations were discovered and stitched. An ileostomy and multiple colic biopsies were also performed. The postoperative course was complicated by persistent septic ileus due to descending and sigmoid colon leaks, which led to colic resections with end-to-end anastomosis. Rectal aspiration biopsies were also performed. At 1 month of age, the infant was discharged from the hospital. The ileostomy was closed in two steps at 2 and 5 months of age. A normal sweat test excluded cystic fibrosis. All colic and rectal biopsies revealed nonspecific inflammatory signs and excluded necrotizing enterocolitis and Hirschsprung disease. Nonspecific irregular thinning of muscularis mucosae and muscularis propria were observed in the two resected colic segments. The boy is now a healthy 7-year-old. The incidence of neonatal focal spontaneous colic perforations at term or close to term is unknown but probably very rare. Our department is the neonatal referral center for approximately 14,000 annual births. In the last 10 years (2000-2009), out of 5115 neonatal admissions in our unit, only ten cases have presented a neonatal spontaneous intestinal perforation, seven of ten in very-low-birth-weight infants and three of ten in term or near-term neonates (one with Hirschsprung disease and the two cases reported herein). In the same period, 108 infants suffered from necrotizing enterocolitis, seven of 108 were term infants and 6 out of 7 had a congenital heart disease. The medical literature is poor on the subject of focal spontaneous colic perforations at term; no risk factor is described. The most specific clinical sign seems to be the abdominal distension. The presence of pneumoperitoneum on an abdominal x-ray is the most sensitive paraclinical sign. In case of an intestinal perforation, surgery must be performed quickly. The vital prognosis seems to be good. The objective of this study was to draw pediatricians' attention to focal spontaneous colic perforations in term or close to term newborns. In the cases reported, the diagnostic delays could have been prevented if the entity - with its radiological manifestation - had been well known.

摘要

报告了两例新生儿局灶性自发性结肠穿孔病例。第一名婴儿,孕36 3/7周出生,出生第3天出现哭闹、腹胀和稀便。临床检查发现婴儿轻度烦躁、体温过低(35.7℃),腹部膨隆,肠鸣音减弱。除C反应蛋白水平升高(59mg/L)外,血液检查正常。腹部X线检查最初被误诊为正常。婴儿病情近3天保持稳定。复查最初的X线片后,怀疑有气腹,并通过腹部交叉侧位X线片得以证实。该婴儿开始使用抗生素并接受手术。肉眼可见,除乙状结肠局灶性穿孔外,整个肠道正常,穿孔处进行了缝合。全层结肠活检显示局灶性血管扩张,但坏死性小肠结肠炎或先天性巨结肠病检查结果为阴性。婴儿恢复很快。她现在是一名健康、正常的3岁儿童。第二名婴儿,孕38 5/7周出生,出生后第1至2天出现与缓慢进展性肠梗阻相关的感染临床症状。胸部和腹部X线检查最初被误诊为正常。随后出现明显败血症。复查最初的X线片后,怀疑有气腹,并通过腹部交叉侧位X线片得以证实。该婴儿接受了手术。肉眼可见,小肠正常,升结肠和横结肠扩张,降结肠和乙状结肠狭窄。发现三处盲肠穿孔并进行了缝合。还进行了回肠造口术和多次结肠活检。术后病程因降结肠和乙状结肠渗漏导致持续性感染性肠梗阻而复杂化,进而导致结肠切除并进行端端吻合。还进行了直肠抽吸活检。婴儿1个月大时出院。回肠造口术在2个月和5个月时分两步关闭。正常的汗液试验排除了囊性纤维化。所有结肠和直肠活检均显示非特异性炎症征象,排除了坏死性小肠结肠炎和先天性巨结肠病。在两段切除的结肠段中观察到黏膜肌层和固有肌层非特异性不规则变薄。该男孩现在是一名健康的7岁儿童。足月或近足月新生儿局灶性自发性结肠穿孔的发病率尚不清楚,但可能非常罕见。我们科室是每年约14000例分娩的新生儿转诊中心。在过去10年(2000 - 2009年)中,在我们科室收治的5115例新生儿中,仅10例出现新生儿自发性肠穿孔,其中10例中有7例为极低体重儿,10例中有3例为足月或近足月新生儿(1例患有先天性巨结肠病,以及本文报告的2例)。同期,108例婴儿患有坏死性小肠结肠炎,108例中有7例为足月儿,7例中有6例患有先天性心脏病。医学文献中关于足月局灶性自发性结肠穿孔的内容较少;未描述危险因素。最具特异性的临床体征似乎是腹胀。腹部X线片上出现气腹是最敏感的辅助检查体征。一旦发生肠穿孔,必须尽快进行手术。生命预后似乎良好。本研究的目的是引起儿科医生对足月或近足月新生儿局灶性自发性结肠穿孔的关注。在报告的病例中,如果对该病及其放射学表现有充分了解,诊断延迟本可避免。

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